The treatment of musculoskeletal neoplasms and infection is usually based on an initial diagnostic biopsy.Prior to biopsy, a hypothesis should be formed about the most likely diagnosis and a differential diagnosis. These deliberations should consider whether the lesion is a primary benign or malignant tumour, a metastasis, a haematological problem or an infection.A tactical plan should be developed which evaluates the necessity, the risk, the approach and finally defines the technique of biopsy most likely to achieve a representative result in the clinical case.In developing this technical approach, the pitfalls should be anticipated, i.e. inadequate sampling, difficulty of pathological interpretation and contamination.The tactical approach should be developed in conjunction with a multi-disciplinary team together with appropriate pre-biopsy imaging.Cite this article: EFORT Open Rev 2017;2:51–57. DOI: 10.1302/2058-5241.2.160065
SJS is clinically and radiologically heterogeneous. The causes of heterogeneity are not known yet but are likely to include both different mutations at the SJS locus on chromosome 1 and the presence of a second SJS locus. A tentative clinico-radiological classification can be useful for the characterization of patients and the development of genotype-phenotype correlations.
Purpose Therapy of vitamin D-resistant hypophosphatemic rickets (VDXLR) consists of oral phosphate and vitamin D supplements. Bone deformities, pain, and small stature can occur even in children with good compliance, requiring surgical correction and bone lengthening. However, only few surgical reports are available. Methods Twelve patients (three males) with VDXLR were followed at our institution. Median age at diagnosis was 3 9/12 years (range, birth to 11 10/12) with a follow-up period of 7 8/12 years (1 9/12-30) and age at last follow-up of 13 6/12 years (2-30). Eight patients underwent surgical correction, three of them in combination with bone lengthening. The corrections were performed at the end of growth in three patients. Clinical endpoints were height, leg axis, and pain. Results Single bilateral surgical correction was performed in six patients; one patient each had three and five corrections. Bone lengthening was performed in three patients. At last follow-up, the height of seven operated patients was within normal range. In addition, leg axis was normalized in six patients with mild genua vara in two. Only one patient complained of intermittent pain. Bone healing was excellent; surgical complications were rare. There was no radiological evidence of degenerative arthropathy.Conclusions Medical treatment remains the main pillar of therapy in children with VDXLR. In case of bone deformity, surgery can safely be performed, independent of age or bone maturation. All patients were satisfied with the results of axial corrective surgery and bone lengthening, and in the majority only one corrective intervention was needed.
Degenerative changes resembling hemophilic arthropathy may be a complication of synovial hemangioma in the knee. It is thought that arthropathy is caused by repeated bleeding episodes similar to the joint disease in hemophilia. Four children aged 4 to 9 years at surgery were treated by anterior open synovectomy for intra-articular synovial hemangiomas. At open surgery the cartilage of all patients showed changes on the surface with yellow hematin staining in all cases, without ulceration in three patients but one, who showed severe degenerative changes at age 9 years. At 15 months follow-up the patient with severe degenerative changes had severely impaired flexion and mild extension deficit. The three others were asymptomatic at 1 to 6 years follow-up. The magnetic resonance images were typical and can be considered pathognomonic; however, the referral diagnosis included the correct diagnosis in none of them. Treatment should be initiated as early as possible to reduce the risk of damage to the cartilage. Treatment by complete open synovectomy in these four patients was performed without significant bleeding problems, and no recurrence was seen.
Definitive epiphysiodesis using this modified Canale technique is a safe, minimal invasive method to correct leg length discrepancy and angular deformities if preoperative planning is performed properly.
Osteomyelitis sclerosans Garré is still not completely understood. In spite of the fact that it is caused by bacterial infection, in most cases no bacterial growth can be discovered in culture. Furthermore, it remains open whether the chronic process is maintained by low-grade persistent infection or is maintained by itself after sterilization of the infection. Newer studies that may prove the presence of bacteria, e.g., by PCR, may elucidate the causes in the future. Sclerosing bone disease, sometimes multifocal, which present the same clinical, radiographic and histological features as chronic sclerosing osteomyelitis may be associated with diseases such as palmoplantar pustulosis, colitis ulcerosa, Crohn's disease, etc. and are reported as SAPHO (synovitis acne pustulosis hyperostosis osteitis) syndrome. In these diseases bacterial growth is almost always negative. However, temporary improvement under antibiotic therapy may be observed. A relationship between typical isolated chronic sclerosing osteomyelitis and SAPHO syndrome may exist, but the diseases should at present be differentiated. Neoplasms such as osteoid osteoma, Ewing sarcoma, or eosinophilic granuloma may simulate primary sclerosing osteomyelitis, and pathological examination of peripherally taken specimens may appear to be compatible with the diagnosis of sclerosing osteomyelitis. It should be appreciated that malignant tumors--even though this is the exception--can present a chronic, mild picture. Treatment of osteomyelitis sclerosans Garré needs be determined on an individual basis. A conservative approach with antibiotics usually leads only to temporary pain relief. The biopsy with opening of the medullary canal is sometimes sufficient for healing, while in extreme situations only segmental or even complete resection of the diseased bone case result in a permanent cure.
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